Langerhans Cell Histiocytosis Treatment - Late Disease and Treatment Effects of Childhood LCH
Liver disease may lead to sclerosing cholangitis, which rarely responds to any treatment other than liver transplantation.
Dental problems characterized by loss of teeth have been significant for some patients, usually related to overly aggressive dental surgery.
Bone marrow failure secondary to LCH or from therapy is rare and is associated with a higher risk of malignancy. Patients with LCH have a higher-than-normal risk of developing secondary cancers.[12,13] Leukemia (usually acute myeloid) occurs after treatment, as does lymphoblastic lymphoma. Concurrent LCH/malignancy has been reported in a few patients, and some patients have had their malignancy first, followed by development of LCH. Three patients with T-cell acute lymphoblastic leukemia (T-ALL) and aggressive LCH, for which the two disorders had shared markers of clonality, have been reported.[14,15] One study reported two cases in which clonality with the same T-cell receptor gamma genotype was found. The authors of this study emphasized the plasticity of lymphocytes developing into Langerhans cells. In the second study, one patient with LCH after T-ALL who had the same T-cell receptor gene rearrangements and activating mutations of the NOTCH1 gene was described.
An association between solid tumors and LCH has also been reported. Solid tumors associated with LCH include retinoblastoma, brain tumors, hepatocellular carcinoma, and Ewing sarcoma.
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- Nanduri V, Tatevossian R, Sirimanna T: High incidence of hearing loss in long-term survivors of multisystem Langerhans cell histiocytosis. Pediatr Blood Cancer 54 (3): 449-53, 2010.
- Nanduri VR, Lillywhite L, Chapman C, et al.: Cognitive outcome of long-term survivors of multisystem langerhans cell histiocytosis: a single-institution, cross-sectional study. J Clin Oncol 21 (15): 2961-7, 2003.
- Mittheisz E, Seidl R, Prayer D, et al.: Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis. Pediatr Blood Cancer 48 (1): 50-6, 2007.
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- Braier J, Ciocca M, Latella A, et al.: Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis. Med Pediatr Oncol 38 (3): 178-82, 2002.
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- Egeler RM, Neglia JP, Aric� M, et al.: The relation of Langerhans cell histiocytosis to acute leukemia, lymphomas, and other solid tumors. The LCH-Malignancy Study Group of the Histiocyte Society. Hematol Oncol Clin North Am 12 (2): 369-78, 1998.
- Feldman AL, Berthold F, Arceci RJ, et al.: Clonal relationship between precursor T-lymphoblastic leukaemia/lymphoma and Langerhans-cell histiocytosis. Lancet Oncol 6 (6): 435-7, 2005.
- Rodig SJ, Payne EG, Degar BA, et al.: Aggressive Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. Am J Hematol 83 (2): 116-21, 2008.